eMedicine Specialties > Dermatology > Diseases of the Adnexa

Graham-Little-Piccardi-Lasseur Syndrome: Differential Diagnoses & Workup

Author: Scott Richard Albert Walsh, MD, PhD, Assistant Professor, Program Director, Department of Dermatology, University of Toronto, Sunnybrook Health Sciences Centre
Coauthor(s): Patricia T Ting, MSc, MD, Dermatology Resident, Division of Dermatology and Cutaneous Sciences, Department of Medicine, University of Alberta; Neil Shear, MD, Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada
Contributor Information and Disclosures

Updated: Oct 13, 2009

Differential Diagnoses

Alopecia Mucinosa
Lichen Planus
Lupus Erythematosus, Discoid
Pityriasis Rubra Pilaris
Pseudopelade, Brocq
Sarcoidosis

Other Problems to Be Considered

Folliculitis decalvans
Keratosis pilaris et atrophicans

Workup

Laboratory Studies

  • Some epidemiological studies describe a loose association between mucocutaneous lichen planus and hepatitis C. However, this association has not yet been described in case reports of Graham-Little-Piccardi-Lasseur syndrome (GLPLS).
  • Initial investigations may include antinuclear antibodies (ANA), antiextractable nuclear antigens (AENA), hepatitis B and C serology, and liver function tests to exclude other associated systemic causes of cicatricial alopecia.

Imaging Studies

  • No imaging studies are required for Graham-Little-Piccardi-Lasseur syndrome (GLPLS).

Procedures

  • Punch biopsies of the scalp parallel to hair shaft growth (or directed at 45° angle in African Americans) may confirm the presence of cicatricial alopecia. Multiple biopsy samples may be required for transverse (horizontal) and vertical sections, and immunohistochemistry. Histologically, the end-stage lesions in Graham-Little-Piccardi-Lasseur syndrome (GLPLS) are indistinguishable from those of pseudopelade of Brocq, discoid lupus erythematosus, folliculitis decalvans, frontal fibrosing alopecia, and other forms of cicatricial alopecia.
  • A skin biopsy of associated follicular papules may reveal the presence of histologic findings that correlate with the triad of GLPLS.
  • The presence of noncicatricial alopecia in the axilla and groin can usually be diagnosed clinically, and further skin biopsies are not necessary.

Histologic Findings

In the early stage, an inflammatory perifollicular lichenoid infiltrate can be observed and is often associated with the infundibuloisthmic (bulge) region of the follicle.8 This infundibuloisthmic region contains stem cells responsible for regenerating the lower two thirds of the hair follicle, which is nonpermanent. The bulb region is spared. In end-stage Graham-Little-Piccardi-Lasseur syndrome (GLPLS), atrophic dermis and fibrosed and empty hair shafts can be seen. One additional histological finding is keratinous follicular plugs with loss of sebaceous glands.

A lichenoid lymphocytic infiltrate composed of mostly CD8 and CD4 T cells may irreversibly damage stem cells and hair follicles. Profibrogenic cytokines such as interleukin (IL)–4 and IL-6, transforming growth factor (TGF)–beta, and interferon (IFN)–gamma have been reported in association with lichen planopilaris of the scalp.9

Direct immunofluorescence studies have reported nonspecific immunoglobulin M, and occasionally immunoglobulin G and immunoglobulin A, at the hair follicle infundibulum and isthmus, as well as linear fibrinogen deposition along the dermoepidermal junction.10

Histopathology of follicular papules
Findings include (1) lichenoid lymphocytic infiltrate in the upper dermis, (2) hyperkeratosis and focal hypergranulosis, (3) acanthosis with occasional saw-toothed rete ridges, and (4) dyskeratotic keratinocytes (Civatte or colloid bodies).

More on Graham-Little-Piccardi-Lasseur Syndrome

Overview: Graham-Little-Piccardi-Lasseur Syndrome
Differential Diagnoses & Workup: Graham-Little-Piccardi-Lasseur Syndrome
Treatment & Medication: Graham-Little-Piccardi-Lasseur Syndrome
Follow-up: Graham-Little-Piccardi-Lasseur Syndrome
Multimedia: Graham-Little-Piccardi-Lasseur Syndrome
References
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References

  1. Graham-Little EG. Folliculitis decalvans et atrophicans. Br J Dermatol. 1915;27:183-5.

  2. Viglizzo G, Verrini A, Rongioletti F. Familial Lassueur-Graham-Little-Piccardi syndrome. Dermatology. 2004;208(2):142-4. [Medline].

  3. Ghislain PD, Van Eeckhout P, Ghislain E. Lassueur-Graham Little-Piccardi syndrome: a 20-year follow-up. Dermatology. 2003;206(4):391-2. [Medline].

  4. Bardazzi F, Landi C, Orlandi C, Neri I, Varotti C. Graham Little-Piccardi-Lasseur syndrome following HBV vaccination. Acta Derm Venereol. Jan 1999;79(1):93. [Medline].

  5. Rodriguez-Bayona B, Ruchaud S, Rodriguez C, et al. Autoantibodies against the chromosomal passenger protein INCENP found in a patient with Graham Little-Piccardi-Lassueur syndrome. J Autoimmune Dis. Jan 12 2007;4:1. [Medline].

  6. Rebora A, Rongioletti F, Drago F, Parodi. Lichen planus as a side effect of HBV vaccination. Dermatology. 1999;198(1):1-2. [Medline].

  7. Vega Gutierrez J, Miranda-Romero A, Perez Milan F, Martinez Garcia G. Graham Little-Piccardi-Lassueur syndrome associated with androgen insensitivity syndrome (testicular feminization). J Eur Acad Dermatol Venereol. Jul 2004;18(4):463-6. [Medline].

  8. Mobini N, Tam S, Kamino H. Possible role of the bulge region in the pathogenesis of inflammatory scarring alopecia: lichen planopilaris as the prototype. J Cutan Pathol. Nov 2005;32(10):675-9. [Medline].

  9. Moretti S, Amato L, Massi D, Bianchi B, Gallerani I, Fabbri P. Evaluation of inflammatory infiltrate and fibrogenic cytokines in pseudopelade of Brocq suggests the involvement of T-helper 2 and 3 cytokines. Br J Dermatol. Jul 2004;151(1):84-90. [Medline].

  10. Horn RT Jr, Goette DK, Odom RB, Olson EG, Guill MA. Immunofluorescent findings and clinical overlap in two cases of follicular lichen planus. J Am Acad Dermatol. Aug 1982;7(2):203-7. [Medline].

  11. Bottoni U, Innocenzi D, Carlesimo M. Treatment of Piccardi-Lassueur-Graham Little syndrome with cyclosporin A. Eur J Dermatol. 1995;5:216-9.

  12. Mirmirani P, Willey A, Price VH. Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol. Oct 2003;49(4):667-71. [Medline].

  13. Boyd AS, King LE Jr. Thalidomide-induced remission of lichen planopilaris. J Am Acad Dermatol. Dec 2002;47(6):967-8. [Medline].

  14. George SJ, Hsu S. Lichen planopilaris treated with thalidomide. J Am Acad Dermatol. Dec 2001;45(6):965-6. [Medline].

  15. Jouanique C, Reygagne P, Bachelez H, Dubertret L. Thalidomide is ineffective in the treatment of lichen planopilaris. J Am Acad Dermatol. Sep 2004;51(3):480-1. [Medline].

  16. Büyük AY, Kavala M. Oral metronidazole treatment of lichen planus. J Am Acad Dermatol. Aug 2000;43(2 Pt 1):260-2. [Medline].

  17. Amato L, Massi D, Berti S, Moretti S, Fabbri P. A multiparametric approach is essential to define different clinicopathological entities within pseudopelade of Brocq. Br J Dermatol. Mar 2002;146(3):532-3. [Medline].

  18. Bianchi L, Paro Vidolin A, Piemonte P, Carboni I, Chimenti S. Graham Little-Piccardi-Lassueur syndrome: effective treatment with cyclosporin A. Clin Exp Dermatol. Sep 2001;26(6):518-20. [Medline].

  19. Chieregato C, Zini A, Barba A, Magnanini M, Rosina P. Lichen planopilaris: report of 30 cases and review of the literature. Int J Dermatol. May 2003;42(5):342-5. [Medline].

  20. Mirmirani P, Willey A, Headington JT, Stenn K, McCalmont TH, Price VH. Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants. J Am Acad Dermatol. Apr 2005;52(4):637-43. [Medline].

  21. Poblet E, Jimenez F, Pascual A, Pique E. Frontal fibrosing alopecia versus lichen planopilaris: a clinicopathological study. Int J Dermatol. Apr 2006;45(4):375-80. [Medline].

  22. Somani N, Bergfeld WF. Cicatricial alopecia: classification and histopathology. Dermatol Ther. Jul-Aug 2008;21(4):221-37. [Medline].

  23. Waldorf DS. Lichen planopilaris. Histopathologic study of disease. Progression to scarring alopedia. Arch Dermatol. Jun 1966;93(6):684-91. [Medline].

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Further Reading

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Keywords

Graham-Little-Piccardi-Lasseur syndrome, GLPLS, Graham-Little-Feldman syndrome, Lassueur-Graham-Little-Piccardi syndrome, Piccardi-Lassueur-Graham-Little syndrome, Graham-Little-Piccardi-Lassueur syndrome, follicular lichen planus, LP, lichen spinulosus et folliculitis decalvans, lichen planopilaris, alopecia, cheratosi spinulosa, keratotic spinulosa, lichenoid dermatosis, hair loss, GLPL syndrome

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Contributor Information and Disclosures

Author

Scott Richard Albert Walsh, MD, PhD, Assistant Professor, Program Director, Department of Dermatology, University of Toronto, Sunnybrook Health Sciences Centre
Scott Richard Albert Walsh, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Canadian Dermatology Association, International Society of Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Patricia T Ting, MSc, MD, Dermatology Resident, Division of Dermatology and Cutaneous Sciences, Department of Medicine, University of Alberta
Patricia T Ting, MSc, MD is a member of the following medical societies: Alberta Medical Association, Canadian Dermatology Association, and Canadian Medical Association
Disclosure: Nothing to disclose.

Neil Shear, MD, Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada
Neil Shear, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Franklin Flowers, MD, Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, University of Florida College of Medicine
Franklin Flowers, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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